Reoperation for hyperparathyroidism in multiple endocrine neoplasia type 1

Kivlen, Maryann H.; Bartlett, David L.; Libutti, Steven K.; Skarulis, Monica C.; Marx, Stephen J.; Simonds, William F.; Weinstein, Lee S.; Jensen, Robert T.; McCart, J. Andrea; Naik, Arpana; Kranda, Karen; Brennan, Murray F.; Norton, Jeffrey A.; Fraker, Douglas L.; Alexander, H. Richard

doi:10.1067/msy.2001.118379

Cited by 66 publications

(56 citation statements)

References 18 publications

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“…However, other reports suggest that there can be a significant false positive rate (i.e., >50% drop in PTH when resection of parathyroid tissue is inadequate) in patients with multigland disease (up to 50% [26]) and a small false negative rate using rapid PTH assays. Kivlen et al [27] reported that the intraoperative PTH assay was of use in 14 patients with MEN 1 undergoing reoperative surgery; however, false positive results were obtained in two patients. To reduce the risk of false positive results in MEN 1 patients, it has been suggested that more stringent criteria should be applied, such as an 80% reduction in PTH prior to accepting adequate excision of parathyroid tissue [28].…”

Section: Total Parathyroidectomymentioning

confidence: 95%

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Primary hyperparathyroidism in MEN 1 – how radical should surgery be?

Hubbard

Sébag

Maweja

et al. 2002

Langenbeck’s Arch Surg

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Primary hyperparathyroidism is the most common manifestation of MEN 1 syndrome. The management of these patients is complex due to the underlying disease process, which predisposes patients to persistent and recurrent disease. The surgical treatment of patients with MEN 1 and hyperparathyroidism can therefore be considered to be palliative in nature. The basic principles of surgery include (1) obtaining and maintaining normocalcaemia for the longest time possible, avoiding persistent/recurrent hypercalcaemia, (2) avoiding surgically induced hypocalcaemia, and (3) facilitating future surgery for recurrent disease. Two approaches have been described as the best practice for patients with hyperparathyroidism in MEN 1: subtotal parathyroidectomy, leaving a remnant of no more than 60 mg in the neck, and total parathyroidectomy with immediate autotransplantation of 10-20 1 mm(3) pieces of parathyroid tissue. Both approaches should be combined with efforts to exclude supernumerary glands and ectopic parathyroid tissue by including resection of fatty tissue from the central neck compartment and thymectomy in all patients. Cryopreservation of parathyroid tissue should be performed whenever facilities are available. In patients with persistent or recurrent disease, an attempt to obtain total elimination of cervical parathyroid tissue is justified, combined with cryopreservation of parathyroid tissue. As radical as surgery is for hyperparathyroidism in MEN 1, the surgeon must take steps to avoid permanent hypoparathyroidism, which in young patients may be worse than the disease itself.

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Section: Total Parathyroidectomymentioning

confidence: 95%

“…A large series of reoperations for persistent and recurrent hyperparathyroidism in MEN 1 patients has recently been reported [27]. Seventy-five patients underwent 79 neck explorations and 12 autograft removals.…”

Section: Reoperative Surgerymentioning

confidence: 99%

Primary hyperparathyroidism in MEN 1 – how radical should surgery be?

Hubbard

Sébag

Maweja

et al. 2002

Langenbeck’s Arch Surg

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“…Even though controversy exists, the Miami criterion of 50 % drop from the highest pre-excision value of PTH is widely accepted [5] for sporadic PHPT. False-positive [6] and false-negative [7] IOPTH assay reports have been shown to occur in multiglandular disease like MEN1. Because of this, the IOPTH has been controversial in multiglandular disease.…”

Section: Discussionmentioning

confidence: 99%

Familial Isolated Hyperparathyroidism: Role of Intra Operative Parathormone Assay

Pradeep

Ramalingam

2012

Indian J Surg

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Primary Hyperparathyroidism (PHPT) has been reported to occur in members of same family either alone or in syndromic association. We report a family of patients with multi glandular disease wherein we have successfully used intra operative PTH (IOPTH) to assess the completeness of resection. Three members of one family were affected (One male and two females). Two of them had symptomatic disease whereas one was asymptomatic. Since the genetic studies are not available we used a combination of radiological, clinical and laboratory findings to rule out the other components of MEN syndromes. Extent of surgery in familial isolated hyperparathyroidism (FIHP) is controversial. Hence for confirmation of adequate parathyroid tissue resection we used IOPTH. Role of IOPTH has been well established in sporadic PHPT but controversial in multi glandular syndromes. IOPTH was successfully used to confirm the excision and establish cure.

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“…The PHPT in MEN1, as compared to the sporadic form, has an earlier age of onset (20)(21)(22)(23)(24)(25) years, compared to 55-60 years), exhibits an equal male to female ratio (compared to a female preponderance in sporadic PHPT), and involves all four parathyroid glands (diffuse hyperplasia or multiple adenomas), rather than a single one (solitary adenomas) [ 8 , 9 ]. Noteworthy is the increased frequency of supernumerary (up to 20 %) and ectopic parathyroid glands in MEN1 patients, usually localized within the thyroid gland, in the anterior mediastinum, or exceptionally in the pericardium [ 18 ]. Although the MEN1 gene is a tumor suppressor, parathyroid carcinoma may be diagnosed in a small percentage of patients [ 19 ].…”

Section: Clinical Features and Diagnosismentioning

confidence: 98%

The Multiple Endocrine Neoplasia Syndromes

Grozinsky‐Glasberg

Gross

2015

Neuroendocrine Tumours

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The term multiple endocrine neoplasia (MEN) refers to hereditary neoplastic disorder involving more than one endocrine organ and includes the MEN type 1 (MEN1), the MEN type 2 (MEN2), and the familial medullary thyroid carcinoma (FMTC). The estimated frequency of each of these syndromes is around 1 in 30,000 and is characterized by complete penetrance with variable expression [ 1 , 2 ]. The recent progress in our knowledge of both the molecular and the clinical genetics of these syndromes has improved the clinical management of the patients [ 3 ]. On the one hand, the MEN1 gene, a tumor suppressor gene responsible of MEN1 syndrome, is involved in the regulation of several cell functions (e.g., DNA replication and repair and transcriptional machinery). On the other hand, the RET proto-oncogene encodes for a receptor tyrosine kinase protein whose expression is fundamental for appropriate migration, development, and differentiation of neuroendocrine cells originating from neural crest [ 4 ]. Currently, DNA testing allows the early identifi cation of germline mutations in presymptomatic mutant gene carriers in the MEN syndromes [ 5 ]. Consequently, an early identifi cation of the MEN-associated neoplasms and the genotype-phenotype correlation improve the outcome and the quality of life for affected subjects [ 6 ].

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Reoperation for hyperparathyroidism in multiple endocrine neoplasia type 1

Cited by 66 publications

References 18 publications

Primary hyperparathyroidism in MEN 1 – how radical should surgery be?

Primary hyperparathyroidism in MEN 1 – how radical should surgery be?

Familial Isolated Hyperparathyroidism: Role of Intra Operative Parathormone Assay

The Multiple Endocrine Neoplasia Syndromes

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